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[Translated article] An Update on EBV-related Cutaneous Lymphoproliferative Disorders: a Systematic Review.

S García-González¹, L Prieto-Torres², M García-García³

¹Servicio de Dermatología, Hospital Clínico Universitario Lozano Blesa, Zaragoza, Spain.

Actas Dermo-Sifiliograficas

|February 12, 2025

Summary

Epstein Barr virus (EBV) positive lymphoproliferative disorders (LPD) with skin involvement range from indolent to aggressive lymphomas. This review details B- and T-cell EBV+ LPD characteristics, aiding diagnosis and avoiding overtreatment.

Keywords:

Epstein Barr Virus-Positive B-cell lymphomas Epstein Barr Virus-Positive B-cell lymphoproliferative disorders Epstein Barr Virus-Positive T-cell lymphoproliferative disorder Linfomas B virus de Epstein Barr positivo Proceso linfoproliferativo T virus de Epstein Barr positivo Procesos linfoproliferativos B virus de Epstein Barr positivo Revisión sistemática Systematic review

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Isolation and Quantification of Epstein-Barr Virus from the P3HR1 Cell Line

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Area of Science:

Hematology
Dermatology
Oncology

Background:

Epstein Barr virus (EBV) positive lymphoproliferative disorders (LPD) encompass rare cutaneous entities with variable clinical behavior.
Classifications of hematological malignancies have evolved since the 2018 WHO-EORTC guidelines, impacting the understanding of EBV+ LPD.
B-cell EBV+ LPD predominantly affect immunocompromised individuals, while T-cell variants are more common in specific global regions.

Approach:

This systematic review synthesizes clinical, histological, immunophenotypic, and molecular data of EBV+ LPD affecting the skin.
It examines both primary cutaneous lymphomas and systemic lymphomas with cutaneous manifestations.
The review discusses diagnostic criteria and differential diagnoses for dermatologists and pathologists.

Key Points:

B-cell EBV+ LPD include EBV-Mucocutaneous Ulcer, lymphomatoid granulomatosis, EBV diffuse large B cell lymphoma, plasmablastic lymphoma, extracavitary primary effusion lymphoma, EBV-positive polymorphic B cell LPD, and post-transplant lymphoproliferative disorders.
T-cell EBV+ LPD encompass extranodal T/NK lymphoma, EBV-positive T-cell centrofollicular lymphoma, and intravascular T/NK-cell lymphoma.
Accurate diagnosis relies on integrating clinical, histological, immunohistochemical, and genetic findings.

Conclusions:

Comprehensive understanding of EBV+ LPD is crucial for accurate diagnosis and appropriate patient management.
Distinguishing between indolent and aggressive forms of EBV+ LPD is essential to prevent overtreatment.
This review provides a scientific basis for the challenging differential diagnosis of cutaneous EBV+ LPD.